Non-immune fetal hydrops: etiology and outcome according to gestational age at diagnosis.

OBJECTIVE: Fetal hydrops is associated with increased perinatal morbidity and mortality. The etiology and outcome of fetal hydrops may differ according to the gestational age at diagnosis. The aim of this study was to evaluate the cause, evolution and outcome of non-immune fetal hydrops (NIFH), according to the gestational age at diagnosis. METHODS: This was a retrospective cohort study of all singleton pregnancies complicated by NIFH, at the Fetal Medicine Unit at St George's University Hospital, London, UK, between 2000 and 2018. All fetuses had detailed anomaly and cardiac ultrasound scans, karyotyping and infection screening. Prenatal diagnostic and therapeutic intervention, gestational age at diagnosis and delivery, as well as pregnancy outcome, were recorded. Regression analysis was used to test for potential association between possible risk factors and perinatal mortality. RESULTS: We included 273 fetuses with NIFH. The etiology of the condition varied significantly in the three trimesters. Excluding 30 women who declined invasive testing, the cause of NIFH was defined as unknown in 62 of the remaining 243 cases (25.5%). Chromosomal aneuploidy was the most common cause of NIFH in the first trimester. It continued to be a significant etiologic factor in the second trimester, along with congenital infection. In the third trimester, the most common etiology was cardiovascular abnormality. Among the 152 (55.7%) women continuing the pregnancy, 48 (31.6%) underwent fetal intervention, including the insertion of pleuroamniotic shunts, fetal blood transfusion and thoracentesis. Fetal intervention was associated significantly with lower perinatal mortality (odds ratio (OR), 0.30 (95% CI, 0.14-0.61); P  0.05). CONCLUSIONS: An earlier gestational age at diagnosis of NIFH was associated with an increased risk of aneuploidy and worse pregnancy outcome, including a higher risk of perinatal loss. Fetal therapy was associated significantly with lower perinatal mortality. © 2020 The Authors. Ultrasound in Obstetrics & Gynecology published by John Wiley & Sons Ltd on behalf of the International Society of Ultrasound in Obstetrics and Gynecology

Nonimmune hydrops foetalis: value of perinatal autopsy and placental examination in determining aetiology

Background: Authors sought to determine the possible factors in the causation of nonimmune hydrops foetalis by perinatal autopsy with placental examination and to reduce the number of cases in which the cause remains elusive.Methods: Twenty five cases of nonimmune hydrops foetalis were identified in about 200 consecutive perinatal autopsies (including placental examination) performed during a 11 year period. The results were correlated with clinical, laboratory and imaging characteristics in an attempt to establish the aetiology.Results: Perinatal autopsy and placental examination confirmed the following aetiologies: cardiovascular causes (8) [isolated (4), syndromic (3) and associated chromosomal (1)], placental causes (5), chromosomal (4) [isolated(3) and associated cardiovascular disease (1)], intrathoracic (3), genitourinary causes (3), infections(1),gastrointestinal lesions (1) and idiopathic causes (1). Placental mesenchymal dysplasia was a unique pathology identified among the placental lesions, which constituted the second most common cause of nonimmune hydrops foetalis. Despite careful examination no cause was identified in one case. In more than 50% of studied cases, autopsy examination either refuted or altered the ultrasound diagnosis completely.Conclusions: The perinatal autopsy in combination with placental study and prenatal imaging represents the most promising tool in the evaluation of aetiology of nonimmune hydrops foetalis. The identification of   a cause for nonimmune hydrops foetalis will provide a better correlation with   recurrence risk and   parental counselling

Prenatal Sonographic Features of Turner Syndrome

Turner syndrome (TS) was first described by Henry Turner in 1938 and was then known to be secondary to karyotypic variation of 45, X in 1959. Most conceptuses with TS spontaneously abort, and only 1% of these embryos survive to term. Fetuses with a pure and complete monosomy X often have more complicated anomalies than those with mosaicism and structural abnormalities in one X chromosome. Ultrasound has been reported to be a reliable tool in the prenatal diagnosis of TS. This article provides an overview of the common sonographic features of fetuses with TS, including cystic hygromas, increased nuchal translucency, non-immune hydrops fetalis, cardiovascular anomalies, urinary anomalies, short femur length, and other rare structural anomalies. Despite that some sonographic markers are transient and may be resolved later in gestation, detection of these markers in early pregnancy should remind obstetric clinicians of the importance of these predictors in TS. The prognosis for cases with TS detected in fetal life is relatively poor. In addition, several diseases may have phenotypic overlap with fetal TS, including non-TS-related cystic hygromas, non-TS-related non-immune hydrops fetalis and Noonan syndrome. Therefore, prenatal recognition of these sonographic features is of great help in karyotypic confirmation, and in appropriate genetic counseling and obstetric treatment

Non-immune hidrops fetalis: Apropos of a case

Presentamos un caso de hidrops fetalis no inmune por trisomía del par 21, diagnosticado a las 31 semanas de gestación. Mediante cordocentesis y estudio cromosómico de linfocitos se llegó al diagnóstico etiológico de síndrome Down. La paciente reingresó a los 6 días del procedimiento con diagnóstico de óbito fetal, produciéndose el parto vaginal electivo al siguiente día. La importancia de este trabajo radica en destacar la factibilidad del diagnóstico citogenético antenatal en nuestro medio mediante técnicas invasivas.We present a case of non-immune hydrops fetalis diagnosed by antenatal ultrasound at 31 weeks of gestation . Fetal karyotyping of blood sample obtained by cordocentesis was abnormal. The fetus died 6 days later. Postmortem examination was compatible with Down syndrome. We report the possibility of antenatal hydrops fetalis diagnosis by invasive techniques

Dizygotic twins concordant for truncus arteriosus

Peer Reviewedhttp://deepblue.lib.umich.edu/bitstream/2027.42/66437/1/j.1399-0004.1991.tb02989.x.pd

A case of fetal hydrops: prenatal diagnosis and neonatal management

Hydrops fetalis (HF) is a serious fetal condition defined as an abnormal fluid accumulation in fetal extravascular compartments and body cavities caused by either immune or non immune conditions. Immune hydrops is caused by fetal hemolysis medi¬ated by circulating maternal antibodies to fetal red blood cell antigens. Its most common determinant is rhesus incompatibility. Systemic Lupus Eritematosus (SLE) is another rare cause of immune fetal hydrops, when the pregnancy is complicated by the presence of a third degree congenital heart block (CHB). The Neonatal Lupus Syndrome occurs with a prevalence of 2%. We reported the case of severe fetal hydrops in a 31 weeks pregnant woman affected by mild maternal D alloimmunization and SLE. Despite fetal hydrops and a mild positive indirect Coombs’ test, the flow-rate study with the Systolic Peak Velocity (PSV) of the MCA excluded a fetal anemia. At birth, blood gas showed a condition of severe metabolic and respiratory acidosis (pH 6.43, pO2 9.9 mmHg, pCO2 206 mmHg, Base Excess (BE) -35 mmol/l, HCO3- 2.7 mmol/l) and a mild anemia (Hemoglobin 10.3 g/dl). ECG revealed a normal sinus rhythm and a CHB was excluded. Despite the critical clinical condition, no cardiorespiratory or neurological adverse outcomes occurred in the newbor

Human parvovirus B19 infection and hydrops fetalis in Rio de Janeiro, Brazil

Formalin-fixed paraffin embedded lung and liver tissue from 23 cases of non immune hydrops fetalis and five control cases, in which hydrops were due to syphilis (3) and genetic causes (2), were examined for the presence of human parvovirus B19 by DNA hybridisation. Using in situ hybridisation with a biotynilated probe one positive case was detected. Using 32P-labelled probes in a dot blot assay format, five further positives were obtained. These were all confirmed as positive by a nested polymerase chain reaction assay. Electron microscopy revealed virus in all these five positive cases. The six B19 DNA positive cases of hydrops fetalis were from 1974, 1980, 1982, 1987 and 1988, four of which occurred during the second half of the year, confirming the seasonality of the disease

Nonimmune hydrops foetalis (NIHF): value of fetal autopsy and comparison with ultrasound findings

Background: Nonimmune hydrops foetalis (NIHF) is a terminal catastrophic event of pregnancy caused by numerous diverse etiology. The aim of this study was to assess the significance of foetal autopsy and to compare the prenatal ultrasound (USG) and foetal autopsy findings in cases of NIHF.Methods: All perinatal autopsies performed at the department of pathology between March 2011-February 2018 were retrospectively reviewed. Of the received 130 autopsies, twenty cases of NIHF were identified, records of which were collected and correlated with maternal medical history, prenatal imaging and autopsy findings.Results: The malformations with hydrops foetalis were classified according to the involved organ system. They were cardiothoracic (7/20 cases), genitourinary (3/20 cases), gastrointestinal lesions (1/20 cases), chromosomal (4/20 cases) and multisystem anomaly/syndromic association (5/20 cases). Complete agreement between USG and autopsy was seen in 8 (40%) cases. In 5 (25%) cases autopsy findings were in total disagreement with USG diagnosis. The rest of the 7 (35%) cases, autopsy revealed additional information and changed the recurrence risk in two cases.Conclusions: Present study demonstrates the high rate of discordancy between USG and autopsy examination in cases complicated by NIHF. Foetal autopsy confirms the USG findings (quality control/audit), adds additional findings or changes the final diagnosis, which helps in redefining the recurrence risk and plausible genetic counselling for future pregnancies. Hence present study underscores the need for autopsy in all cases of NIHF

Hidrops fetal no inmune. A propósito de un caso Nonimmune fetal hydrops. A case report

Non-immune fetal hydrops fetalis is characterized by fluid accumulation in serous cavities and fetal tissues; being a complex pathology linked to a varied etiology, its intrauterine prognosis is uncertain. In spite of the current advances in fetal medicine, there is only a small percentage of therapeutic possibilities, for this reason it is necessary to continue investigating the physiopathological bases of this disease. This case report deals with a 36-year-old female patient with no personal pathological history:  She did not take any medication during pregnancy, except prenatal vitamins, no contact with radiation, no history of blood incompatibility in previous pregnancies, monitored in private practice, where she presented a pathological finding around 13 weeks of gestation in control ultrasound, For this reason she was transferred to a more complex physician, where the decision was made, signed by informed consent to perform therapeutic abortion, procedure without complications, within the findings was corroborated hydrops fetalis, subsequently discharged in good condition. It is concluded that through early ultrasound diagnosis it will be possible to establish an appropriate medical-surgical prognosis for decision making regarding the management of pregnancy.El hidrops fetal no inmune se caracteriza por presentar acumulo de líquido en las cavidades serosas y tejidos fetales; al tratarse de una patología compleja vinculada a una variada etiología, su pronóstico intrauterino es incierto. A pesar de los avances actuales la medicina fetal cuenta nada más con un pequeño porcentaje de posibilidad terapéutica, por tal motivo es necesario seguir investigando las bases fisiopatológicas de esta enfermedad. Este reporte de caso, trato de una paciente de 36 años sin antecedentes personales patológicos:  obstétricos, no ingesta de medicación durante estado gravídico, excepto las vitaminas prenatales, no contacto con radiaciones, ni antecedente de incompatibilidad sanguínea en embarazos anteriores, monitorizada en consultorio particular, donde presento hallazgo patológico alrededor de las 13 semanas de gestación en ecografía de control, motivo por el que fue transferida a facultativo de mayor complejidad, en donde se toma la decisión, firmada por consentimiento informado de realizar aborto terapéutico, procedimiento sin complicaciones, dentro de los hallazgos se corroboro hidrops fetal, posteriormente egreso en buenas condiciones. Se concluye que mediante el diagnostico ecográfico temprano se podrá establecer un pronóstico médico quirúrgico apropiado para la toma de decisiones en cuanto al manejo de la gestación